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11/33. Mixed neuronal-glial tumor of the digestive tract: distinctive entity from gastrointestinal stromal tumor?

    A 53-year-old-woman presenting with pelvic discomfort was found to have a 9.5 cm tumor located in the wall of the ileon. light microscopy showed that the tumor was made of fascicles of plump spindle cells and bizarre epithelioid cells. A cuff of lymphoid cells was also present at the tumor margin. The tumor cells strongly expressed tau protein, neuron-specific enolase, protein green product 9.5 and glial fibrillary acid protein (GFAP), but did not show positive immunostaining for S-100 protein, CD34 or CD117. The tumor showed unequivocal ultrastructural evidence of neural differentiation. Skeinoid fibers were scattered throughout the tumor. This is the first mixed neuronal-glial tumor of the digestive tract to be described in the literature. Such histological and immunohistochemical features could be misinterpreted as features of digestive schwannoma. We suggest that this tumor is distinct from gastrointestinal stromal tumors in lacking CD34 and CD117 expression.
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keywords = spindle cell, spindle
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12/33. A malignant gastrointestinal stromal tumor with osteoclast-like giant cells.

    gastrointestinal stromal tumors (GISTs) are a heterogeneous group of mesenchymal tumors with a wide spectrum of histologic features and consistent expression of c-Kit. We describe an 85-year-old woman who presented with left lower quadrant abdominal pain and was subsequently diagnosed as having a malignant GIST. The tumor was composed of short fascicles of spindle cells. In addition to the presence of tumor giant cells, the tumor also demonstrated many osteoclast-like giant cells, a feature that has not been previously described in the literature. These giant cells expressed histiocytic markers CD68 and alpha(1)-antitrypsin but not c-Kit, a marker for GISTs. Electron microscopy showed no features of smooth muscle differentiation in the giant cells. The possible origin of the osteoclast-like giant cells is discussed in the context of immunohistochemical and ultrastructural characteristics.
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keywords = spindle cell, spindle
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13/33. Reactive nodular fibrous pseudotumor of the gastrointestinal tract and mesentery: a clinicopathologic study of five cases.

    Although the majority of mesenchymal lesions of the gastrointestinal tract are neoplastic in nature, nonneoplastic reactive processes may involve the gastrointestinal tract and mesentery, causing diagnostic confusion with more aggressive neoplasms, such as fibromatosis or gastrointestinal stromal tumors. In this study, we report a series of fibroinflammatory lesions of the gastrointestinal tract that we think represent a relatively cohesive group of tumors and describe the clinical and pathologic features of this entity, which we have termed "reactive nodular fibrous pseudotumor." The tumors affected five patients (four male and one female patient) who ranged in age from 48 to 71 years (mean 56 years). Two patients presented with acute abdominal pain without a significant past medical history, two had incidental lesions discovered during evaluation for other medical conditions, and one was found to have an abdominal mass. Three patients had a history of abdominal surgery. The tumors were multiple in three patients and solitary in two patients. In four cases, at least one of the tumors involved the small intestine or colon, and the lesion was confined to the peripancreatic soft tissue in one case. The tumors were firm, tan-white, ranged in size from 4.3 to 6.5 cm in greatest dimension, and were grossly well circumscribed. All of the lesions were of low to moderate cellularity and composed of stellate or spindled fibroblasts arranged haphazardly or in intersecting fascicles. Three cases had microscopically infiltrative borders. The stroma was rich in collagen, which was wire-like, keloidal, or hyalinized. Intralesional mononuclear cells were sparse but were more numerous peripherally and frequently arranged in lymphoid aggregates. Immunohistochemical stains demonstrated that all of the tumors stained for vimentin, 80% stained for CD117 or muscle specific actin, 60% stained for smooth muscle actin or desmin, and none of the tumors stained for CD34, S-100 protein, or anaplastic lymphoma kinase-1. Follow-up information was available in all cases: four patients had no residual disease following surgical resection (mean follow-up 16.3 months) and one patient who had an incomplete surgical resection had stable disease at 26 months. In summary, we report a series of distinct intraabdominal fibroinflammatory pseudotumors that we have collectively termed "reactive nodular fibrous pseudotumors." These lesions are uncommon and may infiltrate the bowel wall, thereby mimicking primary bowel neoplasms or intraabdominal fibromatosis. Recognition of these nonneoplastic lesions is important, as they pursue a benign clinical course, but may be confused with other mesenchymal neoplasms that require more aggressive treatment.
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ranking = 0.26259683880689
keywords = spindle
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14/33. Retroperitoneal schwannoma with features of gastrointestinal schwannoma. A case report.

    A case of retroperitoneal schwannoma with histological features of gastrointestinal (GI) schwannoma occurring in a 67-year-old woman is reported. The tumor was composed of spindle cells with focal pseudoatypism and it showed several features typical of gastrointestinal-type schwannoma such as lymphocytic infiltration, peripheral cuff of lymphocytes, lack of Antoni A pattern, and absence of thick walled vessels. Immunohistochemically, the tumor showed diffuse reactivity for S100 protein and glial fibrillary acidic protein. The authors discuss a phenotypical similarity of the lesion with GI schwannoma as well as the possible existence of GI-type schwannoma outside the tubal GI tract.
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ranking = 1
keywords = spindle cell, spindle
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15/33. Secondary amyloidosis and gastrointestinal stromal tumors. A case report and discussion of pathogenesis.

    A 69-year-old man presented with a malignant gastrointestinal stromal tumor associated with secondary amyloidosis. The tumor had classic features of a malignant gastrointestinal stromal tumor with interlacing fascicles and whorls of spindled cells, numerous and conspicuous mitotic figures, and extensive coagulative necrosis. The cells stained diffusely for CD117 (c-Kit), confirming the diagnosis of gastrointestinal stromal tumor. The spleen, 1 adrenal gland, and part of the pancreas were removed en block with the stomach. By microscopy, the spleen and adrenal gland were partially replaced with amyloid deposits confirmed by congo red staining, electron microscopy, and immunohistochemistry. In contrast, neither the tumor nor the surrounding vasculature showed amyloid deposition. To our knowledge, this represents only the second case of systemic amyloidosis associated with a gastrointestinal stromal tumor. This case is unique in that extensive, diffuse amyloid deposits were observed in the spleen, adrenal gland, and liver.
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ranking = 0.26259683880689
keywords = spindle
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16/33. Fine needle aspiration appearance of extragastrointestinal stromal tumor. A case report.

    BACKGROUND: gastrointestinal stromal tumors (GISTs) rarely develop outside the digestive tract and in the soft tissues of abdomen and retroperitoneum. Such tumors are designated extra-GISTs (EGISTs). Cytologic and immunocytochemical features of a case of EGIST are reported. CASE: A 54-year-old woman presented with a peritoneal mass, diameter 22 cm, adherent to the omentum and without a connection to the digestive tract. Fine needle aspiration biopsy (FNAB) of the excised tumor showed high cellularity in two patterns: monotonous spindle cells were intermingled with a mildly atypical epithelioid component. Immunocytochemistry performed on cytospins revealed reactivity for c-kit (CD117), CD34 and smooth muscle actin and negativity for S-100. The findings were concordant with a histologic diagnosis of EGIST. CONCLUSION: EGISTs are infrequent neoplasms and can be diagnosed in FNAB samples. The clinical/radiologic setting must be considered together with the cytologic features. Immunocytochemistry is a clue to the diagnosis when it detects c-kit reactivity.
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ranking = 1
keywords = spindle cell, spindle
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17/33. Gastrointestinal autonomic nerve tumours: a case report with ultrastructural and immunohistochemical studies.

    A case of gastrointestinal autonomic nerve tumour with light microscopic, immunohistochemical and ultrastructural examination is reported. The tumour was composed of spindle cells or large cells with clear cytoplasm and showed intense staining for vimentin and focal staining for neuron-specific enolase, chromogranin, synaptophysin, gastrin, P substance and S-100 protein. Ultrastructural examination showed long processes with dense core granules and the absence of features characteristic of other gastrointestinal stromal tumours. In addition we noted small traces of basal lamina and the absence of synaptic vesicles. It seems that the biological behaviour of gastrointestinal autonomic nerve tumours is aggressive but there are too few reports on which to conclude anything about their prognosis. Our findings suggest that tumour has a neuroectodermal differentiation.
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ranking = 1
keywords = spindle cell, spindle
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18/33. Mesenteric fibromatosis with involvement of the gastrointestinal tract. A GIST simulator: a study of 25 cases.

    Mesenteric fibromatosis (MF) and gastrointestinal stromal tumors (GISTs) are distinct lesions, but they often are confused with each other. Correct identification is essential because of their vastly different therapeutic and prognostic connotations. We reviewed 25 cases of MF with involvement of the wall of the gastrointestinal tract and found that GIST was the most common misdiagnosis (13 [52%]). MF was characterized by a spatially homogeneous proliferation of wavy spindle cells without atypia, associated with collagen deposition (often of the keloidal type), and an infiltrative border. Most cases displayed prominent muscular arteries and dilated, thin-walled veins. The mitotic count was relatively low, and no atypical mitotic figures were identified. These features are sufficiently characteristic of MF to permit distinction from GIST on the basis of routinely stained sections in the large majority of the cases, but immunohistochemical analysis provides a supporting role in the few equivocal cases.
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ranking = 1
keywords = spindle cell, spindle
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19/33. Synchronous gastrointestinal and musculoskeletal manifestations of different subtypes of inflammatory myofibroblastic tumor: CT, MRI and pathological features.

    We describe a case of an unusual multicentric appearance of an inflammatory myofibroblastic tumor, consisting of multiple gastrointestinal masses with different growth patterns and simultaneous, distant, musculoskeletal manifestations. CT and MR imaging features demonstrated a different degree of lesion enhancement, which proved histologically to be related to an alternation of predominantly spindle cell areas with a myxoid-vascular IMT subtype. A clear separation of the imaging characteristics of this tumor's subtypes by correlation with the pathology was not possible because of the mixed histologic character of the tumor in all its locations. However, MRI signal in the T2-weighted sequence was lower in the spindle cell variant localized predominantly in the musculoskeletal system, while the gastrointestinal predominantly myxoid-vascular counterparts showed slightly higher signal in the T2-weighted sequence.
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ranking = 2
keywords = spindle cell, spindle
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20/33. Duodenal gangliocytic paraganglioma: a radiological-pathological correlation.

    Duodenal gangliocytic paraganglioma is a rare tumor that characteristically occurs in the second portion of the duodenum and typically presents with gastrointestinal bleeding. Gangliocytic paragangliomas have a characteristic triphasic microscopic appearance with epithelioid cells, spindle cells, and ganglion cells, resulting in a complex histology with features of paraganglioma, carcinoid, and ganglioneuroma. Duodenal gangliocytic paragangliomas have an excellent prognosis after surgical resection but metastatic spread to regional lymph nodes and recurrence may rarely occur. We report a case of duodenal gangliocytic paraganglioma and discuss the radiological and pathological differential diagnosis of this rare entity.
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ranking = 1
keywords = spindle cell, spindle
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